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Hematology Flashcards

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1
Q

What are the “B symptoms” associated with Lymphoma?

A

Fever
Weight Loss
Night Sweats

(These indicate microscopic mets)

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2
Q

What is a common complication seen in patients with thalassemia major having recurrent transfusions?

A

Hemochromatosis

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3
Q

How is Thalassemia Major Transfusion-related Iron overload treated?

A

Deferroximine

Note: Phlebotomy would be counterproductive so cannot do it!

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4
Q

What is the next best step in managing a pt over 50yrs old with microcytic anemia and Negative Stool Guaiac and/or Negative Iron studies ?

A

Colonoscopy

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5
Q

What is the most common cause of Sideroblastic anemia?

A

Alcohol

Note: INH, Pb are other causes

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6
Q

Which Microcytic anemia is most likely to be associated with abnormal peripheral smear?

A

Thalassemia -target cells

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7
Q

Which type of microcytic anemia is most likely to have increased RDW?

A

Iron-deficiency Anemia

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8
Q

Which microcytic anemia is associated with low Ferritin?

A

Iron-deficiency Anemia

Note: Ferritin is also an acute phase reactant

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9
Q

What distinguishing test can be used to dx Fe-deficiency anemia?

A

TIBC-it will be High (b/c there are a lot of binding sites available for carrying iron when iron levels are low)

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10
Q

What is the most accurate test to dx iron-deficiency anemia?

A

Bone marrow Biopsy

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11
Q

Which microcytic anemia will have a high serum iron?

A

Sideroblastic Anemia

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12
Q

What is the most accurate test for sideroblastic anemia?

A

Prussian Blue Stain

see ringed-sideroblasts: iron deposits accumulated in mitochondria of RBC’s

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13
Q

T/F:

Iron studies are abnormal in all forms of thalassemia?

A

False:

All Thalassemias have normal iron studies.

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14
Q

What form of Thalassemia has a high Reticulocyte count?

A

Alpha thalassemia with 3 gene deletion. (Beta 4 tetrad HB formation)

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15
Q

What is the best initial test in patient with alcohol-induced macrocytic anemia?

A

Peripheral Smear (to check for hypersegmented neutrophils)

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16
Q

What is the most common neurological finding associated with B12 deficiency?

A

Peripheral Neuropathy

Note: Any/all types of neurological symptoms can manifest from B12 deficiency

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17
Q

What is the distinguishing feature of Folate deficiency anemia?

A

No neurological Symptoms/findings

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18
Q

What are some causes of Folate deficiency other than decreased intake?

A

Psoriasis (or other desquamating skin conditions)
Pregnancy
Sickle Cell Disease
All these represent very high cell turnover states

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19
Q

If you suspect B12 deficiency and pt has normal B12 levels what is next best step in management?

A

Methylmalonic Acid Level

It will be High in B12 deficiency but not folate deficiency

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20
Q

What metabolite is elevated in both folate and B12 deficiency?

A

Homocysteine

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21
Q

What is next best test once B12 or methylmalonic acid levels are determined?

A

Anti-Intrinsic Factor Ab’s and
Anti-Parietal Cell Ab’s

(these dx pernicious anemia)

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22
Q

What are common lab findings associated with Intravascular hemolysis?

A

Hemoglobinuria (toxic to cells)
Low Haptoglobin

Note: Unconjugated Bilirubin CANNOT filter through glomerular apparatus, it is INSOLUBLE. So dark urine is NOT due to Bbilirubin.

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23
Q

What are two renal complications of direct Hb exposure?

A

Renal Failure/Acute Tubular Necrosis (ATN)

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24
Q

What is the best initial test to dx sickle cell disease?

A

Peripheral Smear

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25
What are the clinical manifestations of Sickle Cell trait?
Hematuria and Isosthenuria
26
What is the next best step in management in a pt with SCD with fever or elevated WBC?
Empiric Antibiotics | DO NOT WAIT FOR CULTURE RESULT
27
What is the etiology of a sudden drop in HCT in a pt with SCD?
Parvovirus B19 causing Aplastic Crisis
28
What is the next step in managing a pt. with SCD who develops a sudden drop in HCT?
Serum Parvovirus B19 PCR | Is PCR not available then do IgM anti-parvovirus Ab
29
What is the best treatment for SCD pt presenting with vision changes, CNS/stroke sx's, acute chest syndrome, priaprism?
Exchange transfusion (Goal is to decrease HbS by 30-40%)
30
What preventative measures should be given to all patients with SCD?
Hydroxyurea Folate replacement Pneumococcal Vaccine
31
What drugs are associated with Autoimmune Hemolysis?
Penicillin Alpha-methyldopa Quinine/quinidine Sulfa Drugs
32
What is the best initial treatment for Autoimmune Hemolysis?
Steroids (Prednisone)
33
What is the next best step in treatment for pt with Autoimmune Hemolysis presenting with recurrent hemolytic episodes?
Splenectomy | remove sight of extravascular RBC destruction. anti-RBC Ab's will be present but the cells will not be destroyed
34
What two conditions are associated with spherocytosis?
Hereditary Spherocytosis | Autoimmune Hemolysis
35
What finding on CBC is associated with HS?
Elevated MCHC
36
What is the most accurate test for dx'ing HS?
Osmotic Fragility Test
37
What is the most accurate test for Paroxysmal Nocturnal Hemoglobinuria?
CD-55 and CD-59 antibody (serum)
38
What is best initial treatment for PNH?
Steroids
39
For transfusion dependent pts, what is best treatment?
Eculizumab (inhibits C-5, preventing complement activation)
40
What is the most likely condition associated with Hemolytic Anemia Venous Thrombosis Pancytopenia
Paroxysmal Nocturnal Hemoglobinuria (PNH)
41
What test is the best initial test for dx'ing G6PD deficiency?
Heinz Body Test (Stain) | Bite Cell present on peripheral smear
42
What is the most common cause of oxidative stress in G6PD deficiency?
Infection
43
What is the most accurate test to Dx G6PD deficiency?
G6PD deficiency level (but should only be done 2 months after presentation. At time of acute presentation, G6PD levels will be normal b/c deficient cells are destroyed first, leaving normal cells behind)
44
What is the best treatment for HS?
Splenectomy
45
What is an alternative treatment for Autoimmune Hemolysis and Cold Agglutinins?
Rituximab (anti-CD20 monoclonal Ab) Note: It is also used to treat Rheumatoid Arthritis as one of the biologic DMARD's
46
How are G6PD def and Pyruvate Kinase Def distinguished?
Pyruvate Kinase def is not induced by oxidative stress.
47
What test should be carried out prior to Dapsone use?
G6PD level or Heinz Bodies/Bite Cells
48
What is the etiology of Aplastic Anemia?
Unknown cause of Killer T-cell induced Pancytopenia
49
What is the treatment for Aplastic Anemia?
Cyclosporine and anti-Thymocyte Immunglobulin (horse-derived anti-Tcell Ab)
50
What are typical symptoms of pancytopenia?(3)
Fatigue (decreased RBC's) Infection (WBC's that do not degranulate) Bleeding (low platelets)
51
What is the most liklely dx for a pt presenting with anemia, thrombocytopenia, leukocytosis with blast predominance in DIC?
AML type M3
52
What is the treatment for AML:M3
Daunorubicin+Cytosine Arabinoside+All Trans Retinoic Acid
53
What is the treatment for ALL?
Daunorubicin+Vincristine+Prednisone +Intrathecal Methotrexate (for prophylaxis against spinal cord mets)
54
What is used to determine who should undergo Bone Marrow Transplant immediately following remission induction?
Cytogenetics Order Karyotype analysis as part of initial work up!
55
What is the most likely dx in a pt presenting with Acute leukemia with a WBC ct >100,000, vision changes, shortness of breath, confusion?
Leukapharesis + Fluids | to rid body of large, excessive wbc's--> treat leukostasis
56
What is the treatment for Chronic Lymphocytic Leukemia (CLL)?
Fludarabine + Rituximab+ Cyclophosphamide
57
What is an alternative treatment for CLL in pts who fail to respond to Fludarabine?
Alemtuzumab
58
What is the mechanism of infection, anemia, and thrombocytopenia associated with late statge CLL?
CLL lymphocytes produce Abnormal/Insufficient Immunoglobulins --> RBC/Platelet attack by abnormal lymphocytes OR reduced amount of IgG --> Hemolysis/ thrombocytopenia and/or recurrent infection
59
What test is used to disinguish between Chronic Myelogenous Leukemia (CML) and Leukemoid Reaction when pt presents with elevated WBC ct with neutrophil predominance?
Lekocyte Alkaline Phosphatase (LAP) High: Leukemoid Rxn Low: CML (a lot of neutrophils that don't work fully)
60
What is the best initial test in work up of pt presenting with fatigue with or without enlarged lymph nodes?
CBC w/ differential: if WBC high with >90% lymphocytes --> CLL if WBC high with >90% Neutrophils --> Do LAP test
61
What is the typical presentation of CML?
Fatigue Early Satiety Abdominal Pain Splenomegaly
62
What is standard treatment for CML in pt positive for Philadelphia Chromosome?
Imatinib (Gleevec) specifically targets the RTK BCR/ABL d/t Philadelphia chromosome. (But this is not a cure!!)
63
What is the best treatment to cure CML?
Bone Marrow transplant (but this is not best initial treatment. Try Imatinibfirst.)
64
What is most common presentation in pt with Multiple myeloma (MM)?
Bone Pain | d/t fracture with normal use
65
What is next best step in pt with recent diagnosis of MM?
Skeletal Survey (look for lytic bone lesions) | Note only do bone scan if skeletal survey is inconclusive
66
What tests should be ordered in workup of pt suspected of MM?
``` Serum and Urine Protein Electrophoresis (BJP) Skeletal Survey (Lytic Lesions) BMP (Chem 7) (Hypercalcemia, BUN/Creat) Peripheral Smear (Rouleaux) Beta2 microglobulin (prognosis) ```
67
What chemo is used to prepare for bone marrow transplant in the treatment of MM?
Adriamycin Vincristine Dexamethasone
68
What is alternative treatment for pt with MM who is over 70 but relatively healthy?
Thalidomide (inhibits TNF) or Lenalidomide or | Bortezomib
69
What other treatments are used for MM?
70
What monoclonal Immunoglobulins are associated with MM?
Monoclonal IgG or IgA
71
What immunoglobulins are associated with MGUS?
IgG (warm Agglutinins)
72
What is the distinguishing features of Waldenstrom Macroglobulinemia-related hyperviscosity ?
``` Blurry vision Confusion Headache Shortness of Breath IgM Spike on SPEP (cold agglutinins) ```
73
What are the mechanisms of renal failure associated with MM?
Hypercalcemia: Nephrocalcinosis Bence Jones Proteins: Clogs glomeruli and Toxic to tubules Immunglobulins: clog glomerular apparatus Hyperuricemia: Toxic to Tubules Amyloid: Damage Nephron
74
What type of bx should be done in a pt who presents with a neck mass that is firm, rubbery, non-tender, non-erythematous, not warm?
Excisional Biopsy
75
What stages of Lymphoma can be treated with Radiation?
Stage I: single LN group involved Stage II: multiple LN groups on same side of Diaphragm (therefore, staging is next step in managing Lymphoma once diagnosed)
76
What chemo therapy agents cause peripheral neuropathy?
Vincristine/Vinblastine
77
Which Chemo agents cause Pulmonary Fibrosis?
Bleomycin/Blusulfan
78
Which pts with Lymphoma are candidates for chemo?
Stage III and IV | Pt. with B symptoms
79
What chemotherapy agent is cardiotoxic?
Adriamycin (daunarubicin)
80
What tests should be done to work up a pt. suspected of Lymphoma?
``` Excisional Bx Chest Xray CT (w/contrast) Head, Chest, Abdomen, Pelvis Bone Marrow Bx anti-CD20 Ag ```
81
What is the treatment for Hodgkins Lymphoma?
Radiation OR Adriamycin Bleomycin Vincristine Dexamethasone
82
What hystologic finding is associated with Hodgkins Lymphoma?
Reed-Sternberg Cells
83
What treatment is typically used to treat Non-Hodgkins Lymphoma?
CHOP and Rituximab (if anti-CD20 Ag present) Cyclophosphamide (hemorrhagic cystitis) Hydroxyadriamycin (cardiotoxicity) Vincristine (peripheral neuropathy) Prednisone (cushing syndrome, psychosis, bone pain/break down)
84
What is next step in management in pt who presents with isolated thrombocytopenia, petechia, and epistaxis without a palpable spleen on exam?
Administer Prednisone | same as for autoimmune hemolysis with Coomb's +
85
If pt. with ITP on Predinose has recurrent episodes of bleeding/thrombocytopenia, what is best treatment ?
Splenectomy | Similar to autoimmune hemolysis
86
What is the next step in a pt who presents with epistaxis, petichiae, melena, intraccranial hemorrhage, menorrhagia, (ie, life threatening bleeding) and platelet count
Administer IVIG or RhoGAM (Fastest way to raise platelet count by occupying macrophages so they cannot destroy paltelets. DO NOT GIVE PLATELETS.)
87
What is the most likely dx in a young healthy female presenting with eoistaxis, petichiae, no spenomegaly, and isolated thrombocytopenia?
Idiopathic Thrombocytopenic Purpura (ITP)
88
What are the distinguishing features associated with Factor XI deficiency?
Deep bleeding associated with rare trauma | Jewish
89
What is a distinguishing feature associated with Factor XII deficiency?
No bleeding, no clotting | Prolonged PTT
90
What is the most likely dx in a pt presenting with Renal failure, thrombocytopenia, and hemolysis following exposure to E. coli O157:87?
Hemolytic Uremic Syndrome
91
What is the best initial treatment for pt. with HUS?
Mild: Symptomatic (self limiting) | If severe: Plasmapheresis
92
What is the next best step in management for a pt presenting with Fever, anemia, thrombocytopenina, renal failure, and neurological findings?
Plasmapharesis (if severe)
93
What is the most accurate test to determine VFW function?
Ristocetin test (platelets will stick together if VWF is working.
94
What is the best initial therapy for Von Willebrand"s Disease?
DDAVP (Desmopressin)
95
What other conditions can DDAVP be used to treat?
Uremic-induced Platelet Destruction | Mild Hemophelia A
96
What is the treatment for severe Hemophelia A or B, or VWD?
Factor VIII replacement | This has both factor VIII and VWF, aka Factor VIII Ag.
97
What coagulation study result is altered in a pt. with Lupus Anticoagulant?
Elevated PTT (that does not decrease)
98
What is the most common cause of unprovoked thromboses with normal PTT?
Factor V Leiden Mutation | Resistant to Protein C
99
What is the most likely dx in a pt with a low PTT that does not increase with heparin bolus?
Antithrombin III deficiency

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